Thursday, May 1, 2008

Rocky Mountain spotted fever (RMSF)

Rocky Mountain spotted fever (RMSF): An acute febrile (feverish) disease initially recognized in the Rocky Mountain states, caused by Rickettsia rickettsii transmitted by hard-shelled (ixodid) ticks. Occurs only in the Western Hemisphere. Anyone frequenting tick-infested areas is at risk for RMSF. The onset of symptoms is abrupt with headache, high fever, chills, muscle pain. and then a rash. The rickettsiae grow within damaged cells lining blood vessels which may become blocked by clots. Blood vessel inflammation (vasculitis) is widespread. Early recognition of RMSF and prompt antibiotic treatment is important in reducing mortality. The first person to describe the disease was an ear, nose and throat specialist, Edward Ernest Maxey. Maxey reported the disease in 1899. Seven years later, a pathologist named Howard Taylor Ricketts showed that it was transmitted by a tick bite. The agent that causes the disease was named for him -- Rickettsia rickettsii. Rocky Mountain spotted fever is also called spotted fever, tick fever, and tick typhus.Common Misspellings: rocky mountain spotted feaver (rmsf)

Syndrome, Aarskog

Syndrome, Aarskog: See: Aarskog-Scott syndrome.

Cancer, lymphoma, non-Hodgkin

Cancer, lymphoma, non-Hodgkin: A lymphoma is a cancer that develops in the lymphatic system. The most common symptom of non-Hodgkin lymphomas is a painless swelling in the lymph nodes in the neck, underarm, or groin. Non-Hodgkin lymphomas are diagnosed with a biopsy of an enlarged lymph node. Follow-up examinations are important after lymphoma treatment. Most relapses occur in the first 2 years after therapy.

Licensed clinical social worker

Licensed clinical social worker: A social worker trained in psychotherapy who helps individuals deal with a variety of mental health and daily living problems to improve overall functioning. A social worker usually has a master's degree in social work and has studied sociology, growth and development, mental health theory and practice, human behavior/social environment, psychology, research methods. Abbreviated L.C.S.W.Common Misspellings: licensed clinincal social worker

Labial sounds

Labial sounds: The lips are not only anatomic features of note, they are organs of speech essential to certain articulations. A sound requiring the participation of one or both lips is a labial (labium in Latin means lip) sound or, simply, a labial. All labials are consonants. There are bilabial sounds such as "p" which involve both lips and labiodental sounds such as "v" which involve the upper teeth and lower lip.

Pedodontics

Pedodontics: Children's dentistry.

Shock, septic

Shock, septic: Shock caused by infection. See also septicemia.

CAT scan, helical

CAT scan, helical: A conventional computerized axial tomography scan (CAT scan or CT scan) is an x-ray procedure which combines many x-ray images with the aid of a computer to generate cross-sectional views and, if needed, three-dimensional images of the internal organs and structures of the body. A CAT scan is used to define normal and abnormal structures in the body and/or assist in procedures by helping to accurately guide the placement of instruments or treatments. A helical CAT scan is a new specialized CAT scan technique that involves continuous movement of the patient through the scanner with the ability to scan faster and with higher definition of internal structures. Helical CAT scanning can permit greater visualization of blood vessels and internal tissues, such as those within the chest cavity. This form of scanner may be particularly helpful in the rapid evaluation of severe trauma injuries, such as those sustained in automobile accidents. A helical CAT scan is also referred to as spiral CAT scan.

Disease, phytanic acid storage

Disease, phytanic acid storage: See Refsum disease.

Plaque, skin

Plaque, skin: A plaque is a broad, raised area on the skin. Because it is raised, it can be felt (palpated). By definition, a skin plaque has a greater surface than its elevation above the skin surface: it is broader than it is high.

Ophthalmic artery

Ophthalmic artery: The ophthalmic artery supplies blood to the eye and adjacent structures of the face. It arises from the internal carotid artery that courses up deep within the front of the neck.

Acrodynia

Acrodynia: Pain in the extremities (the hands and feet).

Disease, sickle cell

Disease, sickle cell: A genetic blood disease due to the presence of an abnormal form of hemoglobin, namely hemoglobin S. Hemoglobin is the molecule in red blood cells that transports oxygen from the lungs to the farthest reaches of the body. Sickle cell disease, also known as sickle cell anemia, affects millions of people throughout the world. It is particularly common among people whose ancestors came from sub-Saharan Africa; Spanish-speaking regions (South America, Cuba, Central America); Saudi Arabia; India; and Mediterranean countries, such as Turkey, Greece, and Italy. In the USA, sickle cell disease occurs in about 1 in every 500 African-American births and 1 in every 1,000-1,400 Hispanic-American births. Sickle cell anemia is caused by an error in a gene that makes the beta globin chain of hemoglobin. The resultant abnormal hemoglobin (sickle hemoglobin) deforms the red blood cells when they are under low oxygen conditions. Children who inherit 2 copies of the sickle gene, one from each parent, have sickle cell anemia. Children who inherit the sickle gene from only one parent do not have the disease, but will carry the sickle cell trait. Individuals with sickle cell trait generally have no symptoms. About 2 million Americans (or 1 in 12 African-Americans) carry the sickle gene. The sickle mutation occurred thousands of years ago. The sickle gene became common in malarious areas because it affords a selective advantage against malaria. In the U.S., Canada, and many other countries where malaria is rare today, the sickle gene can no longer use its anti-malarial advantage. Instead, the sickle gene may be a serious threat to the carrier's children, who may inherit two abnormal sickle hemoglobin genes and have sickle cell anemia. In sickle cell anemia, the hemoglobin molecules tend to aggregate after unloading oxygen. They form long, rod-like structures that force the red cells to assume a sickle shape. Unlike normal red cells, which are usually smooth and deformable, the sickle red cells cannot squeeze through small blood vessels. The sickle cells block little vessels depriving the organs of blood and oxygen. This leads to the periodic episodes of pain and damages the vital organs. Whereas normal red blood cells last about 120 days in the bloodstream, sickle red cells die after only about 10 to 20 days. Because they cannot be replaced fast enough, the blood is chronically short of red blood cells causing anemia -- sickle cell anemia.

Tinea capitis

Tinea capitis: A superficial fungus infection of the skin, affecting the scalp. Also known as ringworm. This disorder occurs most commonly in children, especially those in late childhood and adolescence. It can spread in schools. Tinea capitis appears as scalp scaling that is associated with bald spots (in contrast to seborrhea or dandruff, for instance, which do not cause hair loss).

Macular pucker

Macular pucker: Scar tissue in the macula, the area of the retina responsible for sharp central vision. The scar can blur and distort vision and make straight lines appear wavy. Macular pucker is due most often to age-related shrinkage of the vitreous which pulls away from the retina, causing the retina to scar and wrinkle. Other causes of macular pucker include trauma (from surgery or an eye injury), retinal detachment, inflammation, and problems with the retinal blood vessels. The only treatment is surgery which consists of a vitrectomy (removal of the vitreous) combined with peeling away of the scar tissue. Most patients recover about half of their lost vision, and distortion is significantly reduced. The most common complication of vitrectomy is an increase in the rate of cataract development. Also called epiretinal membrane, retinal wrinkling, premacular fibrosis, and cellophane maculopathy.

Aphasia, amnesic

Aphasia, amnesic: See Anomia.

Virus, pox

Virus, pox: See: Orthopox.

Diastrophic dysplasia

Diastrophic dysplasia: An inherited skeletal disorder involving significantly short stature (dwarfism). Characteristic features at birth include short birth length with short limbs (short-limbed dwarfism), "hitchhiker thumb", and clubfeet. Palatal malformations such as cleft palate or submucous cleft of the palate are present in 50% of patients. There is swelling of the ears in the first days to weeks of life in 80% of children; the swelling then spontaneously subsides but later the ears have a "cauliflower" appearance. Fingers are short and broad and show ulnar deviation (are inclined away from the thumb). The thumb itself has a hitch-hiker-type appearance. There is increased death rate in infancy due to problems breathing, but thereafter people with diastrophic dysplasia have a normal life span. Orthopedic problems are common. The joints can be dislocated, especially the shoulder, elbows, hips, and patellae (knee caps). Flexion contractures of the knees and shoulders are common. Scoliosis (sideways curvature of the spine) is not present at birth but often develops and is progresses particularly in the early teen years. Treatment of the scoliosis includes bracing and occasionally, spinal fusion. Progressive kyphosis (forward bending of the spine) can also occur with what is called subluxation (slippage) which can result in compression of the spinal cord. The average length of babies with diastrophic dysplasia at birth is 16 1/2 inches (42 cms). The average adult height is slightly under 4 feet -- 46-47 inches (118 cms) to be exact. The final adult height is influenced by the presence of scoliosis, hip contractures and knee contractures, and foot deformities. Diastrophic dysplasia is inherited as an autosomal recessive condition meaning that the gene for diastrophic dysplasia is on one of the nonsex chromosomes and average-sized parents have a 1 in 4 (25%) chance of having additional children with diastrophic dysplasia. The gene for diastrophic dysplasia is on chromosome 5q32-q33.1 and is called "diastrophic dysplasia sulfate transporter" (DTDST). Prenatal diagnosis has been performed for diastrophic dysplasia by ultrasound and by molecular DNA diagnosis to detect DTDST. Diastrophic dysplasia was first clearly delineated by Maroteaux and Lamy in France in 1960. The term "diastrophic" was borrowed by Lamy and Maroteaux from geology: diastrophism is the process of bending of the earth's crust by which mountains, continents, ocean basins, etc., are formed. Dysplasia means abnormal in form. Common Misspellings: diastrophic displasia, diastrophic diplasia

Hepatitis, viral

Hepatitis, viral: Liver inflammation caused by viruses. Specific hepatitis viruses have been labeled A, B, C, D, E, F, and G. While other viruses, such as the mononucleosis (Epstein-Barr) virus and cytomegalovirus, can also cause hepatitis, the liver is not their primary target.

TIPS

TIPS: TIPS stands for "transjugular, intrahepatic, portosystemic shunt." It is a shunt (tube) placed between the portal vein which carries blood from the intestines to the liver and the hepatic vein which carries blood from the liver back to the heart. It is used primarily (but not exclusively) in patients with cirrhosis in which the scar tissue within the liver has blocked the flow of blood passing through the liver from the portal vein to the hepatic vein. The blockage increases the pressure in the portal vein leading to an increase in pressure in the portal vein (portal hypertension). As a result of the increase in pressure, blood flows around the liver via small, unimportant veins that connect the portal vein with other veins within the abdomen. These veins enlarge and are referred to as varices. Unfortunately, one of the places varices form is in the stomach and lower esophagus, and these varices have a tendency to bleed massively, frequently causing death from exsanguination. By providing an artificial path for blood traveling from the intestines, through the liver, and back to the heart, TIPS reduces the pressure in the varices and prevents them from rupturing and bleeding. There are several types of shunts that are placed surgically. TIPS is a non-surgical way of placing a portosystemic shunt. The shunt is passed down the jugular vein in the neck by a radiologist using x-ray guidance. The shunt then is inserted between the portal and hepatic veins within the liver.