Jeune's Syndrome Common Terminology :: Nationwide Children's Hospital

Jeune's Syndrome Common Terminology

Most likely every parent that hears the news their child has Jeune's, is likely to find out every single thing they possibly can about the condition. This can be a feat within itself, as many of the terms related to Jeune's Syndrome will be words you will likely not be familiar with. We've compiled a list of the most common words having to do with the conditions.

Use the links below for definitions on each term.

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Achondroplasia: Improper development of cartilage at the ends of the long bones, resulting in a small skeleton.

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Alveolar hypoventilation: Deficient ventilation of tiny, thin-walled, capillary-rich sacs in the lungs where the exchange of oxygen and carbon dioxide takes place that results in reduction in the oxygen content or increase in the carbon dioxide content of the blood or both

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Anterior Mediastinum: The area in the front of the chest behind the breastbone

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Anteriorly: Implies the front of an area (as opposed to the back).

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Anterolateral areas of the rib cage: The area of the ribcage that is behind the arm as it hangs down at rest.

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Apnea: Temporary absence or cessation of breathing

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Areola: The colored ring around the nipple

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Arterial vessel wall thickening: Thickening of the sides of the blood vessels that carry oxygenated blood away from the lungs and heart

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Asphyxia: Inability to breath

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Asphyxiating: Smothering or suffocating so as to cause inability to breath

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Asphyxiating Thoracic Dystrophy: Another term for Jeunes Syndrome which describes the syndrome: Asphyxiation (inability to breath) because of dystrophy (malformation or deformity) of the thorax (chest).

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Aspiration: The taking of foreign matter into the lungs with the respiratory current

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Autosomal recessive disease: Describes an inherited disorder that can be passed on to an offspring from a parent who doesn't have that same disorder

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Barnes Syndrome: A very similar syndrome to Jeunes Syndrome but also associated with a very small voice box and some other findings. The chest wall problem is the same as with Jeunes.

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Bilateral expansion: To surgically enlarge both sides of the chest

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Bilaterally: Both sides

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Broncheomalacia: Disease of the small airtubes that makes them too soft so that they collapse when air tries to leave the lung.



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Calcification: The deposit of bone like material containing calcium- particulary in the bed of ribs when the rib has been removed from the bed.



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Cartilage: A usually translucent somewhat elastic tissue that composes most of the skeleton of vertebrate embryos and except for a small number of structures (as some joints, respiratory passages, and the external ear) is replaced by bone during ossification in the higher vertebrates

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Chest roentegenographs: An X-ray of the chest

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Chest Tube: Surgical insertion of a hollow, flexible drainage tube into the chest. Chest tubes are inserted to drain blood, fluid, or air and allow full expansion of the lungs. The tube is placed between the ribs and into the space between the inner lining and the outer lining of the lung (pleural space).

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Chondrodysplasia: A hereditary skeletal disorder characterized by improper growth of the cartilage portion of the ribs

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Costochondral: Relating to the joint between the boney portion of the rib and the cartilage portion

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CT Scan/Imaging: A special kind of xray study which, with the use of computer analysis, can create 3 dimensional pictures of the chest cage and its contents.

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Diffuse rhonchi: A kind of sound in the chest you can hear with a stethescope that comes from partially blocked small airways

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Dwarfism: Any type of body situation that results in a very small person. Jeunes syndrome is one form of dwarfism.

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Ellis-van Creveld syndrome: Ellis-Van Creveld Syndrome is a rare genetic disorder characterized by short limb dwarfism, additional fingers and/or toes (polydactyly), abnormal development of fingernails and, in over half of the cases, congenital heart defects. This disorder is inherited through an autosomal recessive trait

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Endochondral bone formation in utero: A situation where, before birth, bone is formed where there should be cartilage instead.

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Fractional lung volumes: One of several measurements that can be made from a breathing test that helps to understand the mechanics of breathing in an individual patient.

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Gentamicin aerosols for tracheitis prophylaxis: An antibiotic spray used to help prevent infection in the windpipe.

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Hilar vessels: Large blood vessels going to and from the lung at the root of each lung where it meets the heart.

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Hydrocephalus: Water on the brain that makes for a very large head

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Hypoplastic: Small or underdeveloped

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Intercostal muscles: Any of the short muscles that extend between the ribs filling in the space between them and serving to move the ribs in respiration

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Jeunes Syndrome: A potentially deadly form of congenital dwarfism characterized by a very small chest wall which can restrict the lungs and make breathing difficult or impossible. It may be severe requiring breathing machines in infancy or mild allowing survival into adulthood. In some cases there are associated problems with the kidneys.

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Lateral thoracic expansion: An operation on the chest wall which spreads the ribs apart and creates a bigger cavity inside

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Lung alveolar growth: Growth of the air cells of the lungs

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Lung tissue disease: Disease that effects the tissues of the lung as opposed to the airtubes of the lung or the space surrounding the lung.

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Malacic airway: Any disease involving the airtubes which takes away the normal stiffness which is required to easily move air, and renders them too soft

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Mediastinal structures: Structures in the middle of the chest cavity

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Mediastinum: The region the center of the chest between the lungs which includes the heart, windpipe, swallowing tube and certain glands

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Neonatal: Newborn

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Parietal pleura: The inside lining of the chest wall that lies beside the outside of the lung

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Pectus Carinatum: A deformity of the chest wall characterized my a major protrusion of the breastbone forward; also called “pigeon breast”.

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Pectus Excavatum: A deformity of the chest wall characterized by a major indentation of the breastbone; also called “funnel chest”.

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Pelvis: The collection of bones at the base of the belly that connects to the legs

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Periosteal bridges: A situation where two adjacent ribs heal together to form a bridge of bone between them

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Periosteal elevator: A surgical instrument which is used to scrape off the rib lining from the rib itself.

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Periostium: The bed in which the ribs lie. If the ribs are separated from the rib bed, the rib bed can form more rib.

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Phalanges: Fingers or toes

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Plastic surgery: The branch of surgery concerned with restoration, reconstruction, or improvement of defective, damaged, or missing structures.

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Pleura: Lining of the lung cavity: "visceral pleura" lines the outside of the lung; "parietal pleura" lines the inside of the chest wall

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Pneumonia: An infection of lung tissue

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Polychondrodystrophy: A situation where cartilage is formed improperly in many different parts of the body

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Posteriorly: Situated behind

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Prosthetic material: Any artificial material used for the replacement of missing body parts

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Pulmonary hypertension: High blood pressure in the lungs (as opposed to high blood pressure in the body)

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Pulmonary hypoplasia: Under-development of lung

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Pulmonary parenchymal: Referring to the tissues of the lung as opposed to the airtubes of the lung or the space surrounding the lung.

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Pulmonology: A subspecialty of internal medicine concerned with the study of the respiratory system. It is especially concerned with diagnosis and treatment of diseases and defects of the lungs and airtubes.

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Radiology: A specialty concerned with the use of x-ray and other forms of images in the diagnosis and treatment of disease

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Renal dysfunction: Malfunctioning of the kidneys

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Single lumen endotracheal anesthesia: Anesthesia administered by one single breathing tube, as opposed to a divided tube which can treat the two lungs differently

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Sternal split: A technique to enlarge the chest by dividing the breastbone in the front and spreading it apart.

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Sternum: Breastbone.

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Subcutaneous: Under the skin

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Subperiosteal rib osteotomies: Dividing the rib separately from its underlying rib bed

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Thoracic: Anything to do with the chest or its contents (heart, lung, etc)

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Thoracic cage: Anything to do with the chest gage

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Thoracic surgery: Surgery involving the area of the chest: chest wall, heart, lungs and esophagus

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Thoracotomy: Surgical incision into the chest wall

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Thorax: The chest

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Titanium struts: Small metal bars made of Titanium that can accommodate screws that go into the rib to hold two ends of rib together while they heal.

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Tomography: A highly technical x-ray system with a computer program that puts together many different xrays taken from many different angles and reconstructs a single two-or-three dimensional picture of an area of the body

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Tracheomalacia: Disease of the main windpipe that makes it too soft so that it collapses when air tries to leave the lung.

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Tracheostomy: Surgical construction of an opening in the trachea for the insertion of a catheter or tube to facilitate breathing.

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Transverse incision: A side to side incision (as opposed to an up and down incision

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Ventilator: Breathing machine that moves air into and out of the lungs

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Ventriculoperitoneal shunt: Surgical placement of a plastic tube to drain excess fluid from the brain and divert it into the abdomen, therefore preventing hydrocephalus or "water on the brain"

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