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Asphyxiating Thoracic Dysplasia
Asphyxiating thoracic dysplasia (ATD), also known as Jeune syndrome, is a rare inherited bone growth disorder (autosomal recessive skeletal dysplasia) that primarily affects the thoracic region. It was first described in 1955 by the French pediatrician Mathis Jeune. Common signs and symptoms can include a narrow chest, short ribs, shortened bones in the arms and legs, short stature, and extra fingers and toes (polydactyly). The restricted growth and expansion of the lungs caused by this disorder results in life-threatening breathing difficulties; occurring in 1 in every 100,000-130,000 live births in the United States. People who are affected with this disorder live short lives either only into infancy or early childhood. If they live beyond childhood, breathing problems can improve with age, but there is a possibility of developing severe kidney or heart problems. Several mutations in different genes such as '' IFT80'', ''DYNC2H1'', '' WDR19'', '' IFT140'' and '' TTC21B'' h ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Polydactyly
Polydactyly or polydactylism (), also known as hyperdactyly, is an anomaly in humans and animals resulting in supernumerary fingers and/or toes. Polydactyly is the opposite of oligodactyly (fewer fingers or toes). Signs and symptoms In humans/animals this condition can present itself on one or both hands or feet. The extra digit is usually a small piece of soft tissue that can be removed. Occasionally it contains bone without joints; rarely it may be a complete functioning digit. The extra digit is most common on the ulnar (little finger) side of the hand, less common on the radial ( thumb) side, and very rarely within the middle three digits. These are respectively known as postaxial (little finger), preaxial (thumb), and central (ring, middle, index fingers) polydactyly. The extra digit is most commonly an abnormal fork in an existing digit, or it may rarely originate at the wrist as a normal digit does. The incidence of congenital deformities in newborns is approximately 2 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Dwarfism
Dwarfism is a condition wherein an organism is exceptionally small, and mostly occurs in the animal kingdom. In humans, it is sometimes defined as an adult height of less than , regardless of sex; the average adult height among people with dwarfism is , although some individuals with dwarfism are slightly taller. ''Disproportionate dwarfism'' is characterized by either short limbs or a short torso. In cases of ''proportionate dwarfism'', both the limbs and torso are unusually small. Intelligence is usually normal, and most have a nearly normal life expectancy. People with dwarfism can usually bear children, though there are additional risks to the mother and child dependent upon the underlying condition. The most common and recognisable form of dwarfism in humans (comprising 70% of cases) is achondroplasia, a genetic disorder whereby the limbs are diminutive. Growth hormone deficiency is responsible for most other cases. Treatment depends on the underlying cause. Those ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Methyl Methacrylate
Methyl methacrylate (MMA) is an organic compound with the formula CH2=C(CH3)COOCH3. This colorless liquid, the methyl ester of methacrylic acid (MAA), is a monomer produced on a large scale for the production of poly(methyl methacrylate) (PMMA). Production and properties Given the scale of production, many methods have been developed starting from diverse two- to four-carbon precursors.. Two principal routes appear to be commonly practiced. Cyanohydrin route The compound is manufactured by several methods, the principal one being the acetone cyanohydrin (ACH) route. ACH is produced by condensation of acetone and hydrogen cyanide. The cyanohydrin is hydrolyzed in the presence of sulfuric acid to a sulfate ester of the methacrylamide. Methanolysis of this ester gives ammonium bisulfate and MMA. Although widely used, the ACH route coproduces substantial amounts of ammonium sulfate. :(CH3)2CO + HCN → (CH3)2C(OH)CN :(CH3)2C(OH)CN + H2SO4 → (CH3)2C(OSO3H)C(O)NH ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Median Sternotomy
Median sternotomy is a type of surgical procedure in which a vertical inline incision is made along the sternum, after which the sternum itself is divided using a sternal saw. This procedure provides access to the heart and lungs for surgical procedures such as heart transplant, lung transplant, corrective surgery for congenital heart defects, or coronary artery bypass surgery Coronary artery bypass surgery, also known as coronary artery bypass graft (CABG, pronounced "cabbage") is a surgical procedure to treat coronary artery disease (CAD), the buildup of plaques in the arteries of the heart. It can relieve chest pai .... References {{Cardiac surgery Thoracic surgical procedures Surgical incisions ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Periosteum
The periosteum is a membrane that covers the outer surface of all bones, except at the articular surfaces (i.e. the parts within a joint space) of long bones. Endosteum lines the inner surface of the medullary cavity of all long bones. Structure The periosteum consists of an outer fibrous layer, and an inner cambium layer (or osteogenic layer). The fibrous layer is of dense irregular connective tissue, containing fibroblasts, while the cambium layer is highly cellular containing progenitor cells that develop into osteoblasts. These osteoblasts are responsible for increasing the width of a long bone and the overall size of the other bone types. After a bone fracture, the progenitor cells develop into osteoblasts and chondroblasts, which are essential to the healing process. The outer fibrous layer and the inner cambium layer is differentiated under electron micrography. As opposed to osseous tissue, the periosteum has nociceptors, sensory neurons that make it very sensitiv ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Vertical Expandable Prosthetic Titanium Rib
Vertical Expandable Prosthetic Titanium Ribs (VEPTR) is a product of Synthes for the treatment of childhood deformities of the thorax. It is a special form of a lengthenable rod ("Growing Rod"). An alternative system is the ''USS pediatric'' for older children, also from Synthes. The instrument is used to dilate a too small or too narrow rib thorax in severe thoracic deformity. It consists of a telescopic "titanium rib" in curved form with several holes in a row for fixing in the desired length. A prolongation can be carried out after 6 months. The fixation takes place between two ribs or between a rib and the iliac crest The crest of the ilium (or iliac crest) is the superior border of the wing of ilium and the superiolateral margin of the greater pelvis. Structure The iliac crest stretches posteriorly from the anterior superior iliac spine (ASIS) to the poster .... This results in an indirect erection of the deformed spine, resulting in an increase in the volume of the tho ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Postural Drainage
Postural drainage (PD) is the drainage of lung secretions using gravity. It is used to treat a variety of conditions that cause the build-up of secretions in the lungs. Uses Postural drainage is used to treat any condition that causes the build-up of secretions in bronchopulmonary segments. These include: * bronchiectasis * lung abscesses * cystic fibrosis * atelectasis * chronic obstructive pulmonary disease (COPD) * pneumonia * postoperative lung damage (after some thoracic surgery) * Covid 19 Patients must receive physiotherapy to learn to tip themselves into a position in which the lobe can be drained. Contraindications Postural drainage is often not suitable for infants in the neonatal intensive care unit, who may have lots of equipment attached to them. Postural drainage is more difficult if patients experience poor mobility, poor posture, pain, anxiety, and skin damage, usually requiring adaptations to the technique. Trendelenburg position which is head dow ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Mechanical Ventilation
Mechanical ventilation, assisted ventilation or intermittent mandatory ventilation (IMV), is the medical term for using a machine called a ventilator to fully or partially provide artificial ventilation. Mechanical ventilation helps move air into and out of the lungs, with the main goal of helping the delivery of oxygen and removal of carbon dioxide. Mechanical ventilation is used for many reasons, including to protect the airway due to mechanical or neurologic cause, to ensure adequate oxygenation, or to remove excess carbon dioxide from the lungs. Various healthcare providers are involved with the use of mechanical ventilation and people who require ventilators are typically monitored in an intensive care unit. Mechanical ventilation is termed invasive if it involves an instrument to create an airway that is placed inside the trachea. This is done through an endotracheal tube or nasotracheal tube. For non-invasive ventilation in people who are conscious, face or nasal ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ultrasound
Ultrasound is sound waves with frequencies higher than the upper audible limit of human hearing. Ultrasound is not different from "normal" (audible) sound in its physical properties, except that humans cannot hear it. This limit varies from person to person and is approximately 20 kilohertz (20,000 hertz) in healthy young adults. Ultrasound devices operate with frequencies from 20 kHz up to several gigahertz. Ultrasound is used in many different fields. Ultrasonic devices are used to detect objects and measure distances. Ultrasound imaging or sonography is often used in medicine. In the nondestructive testing of products and structures, ultrasound is used to detect invisible flaws. Industrially, ultrasound is used for cleaning, mixing, and accelerating chemical processes. Animals such as bats and porpoises use ultrasound for locating prey and obstacles. History Acoustics, the science of sound, starts as far back as Pythagoras in the 6th century BC, who ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Thoracic Insufficiency Syndrome
Thoracic insufficiency syndrome is the inability of the thorax to support normal respiration. It is frequently associated with chest and/or spinal abnormalities. Treatment options are limited, but include supportive pulmonary care and surgical options ( thoracoplasty and/or implantation of vertical expandable prosthetic titanium rib Vertical Expandable Prosthetic Titanium Ribs (VEPTR) is a product of Synthes for the treatment of childhood deformities of the thorax. It is a special form of a lengthenable rod ("Growing Rod"). An alternative system is the ''USS pediatric'' for ... (VEPTR) devices). References Respiratory diseases Syndromes {{respiratory-disease-stub ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ciliopathy
A ciliopathy is any genetic disorder that affects the cellular cilia or the cilia anchoring structures, the basal bodies, or ciliary function. Primary cilia are important in guiding the process of development, so abnormal ciliary function while an embryo is developing can lead to a set of malformations that can occur regardless of the particular genetic problem. The similarity of the clinical features of these developmental disorders means that they form a recognizable cluster of syndromes, loosely attributed to abnormal ciliary function and hence called ciliopathies. Regardless of the actual genetic cause, it is clustering of a set of characteristic physiological features which define whether a syndrome is a ciliopathy. Although ciliopathies are usually considered to involve proteins that localize to motile and/or immotile (primary) cilia or centrosomes, it is possible for ciliopathies to be associated with unexpected proteins such as XPNPEP3, which localizes to mitochondri ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Kidney Failure
Kidney failure, also known as end-stage kidney disease, is a medical condition in which the kidneys can no longer adequately filter waste products from the blood, functioning at less than 15% of normal levels. Kidney failure is classified as either acute kidney failure, which develops rapidly and may resolve; and chronic kidney failure, which develops slowly and can often be irreversible. Symptoms may include leg swelling, feeling tired, vomiting, loss of appetite, and confusion. Complications of acute and chronic failure include uremia, high blood potassium, and volume overload. Complications of chronic failure also include heart disease, high blood pressure, and anemia. Causes of acute kidney failure include low blood pressure, blockage of the urinary tract, certain medications, muscle breakdown, and hemolytic uremic syndrome. Causes of chronic kidney failure include diabetes, high blood pressure, nephrotic syndrome, and polycystic kidney disease. Diagnosis of acute f ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
