Terms & Definitions

Glossary of Terms

air-trapping — The retention of a significant amount of air in one's lungs, even after both normal and maximal exhalation.

alveoli — Tiny air sacs at the ends of the bronchial tubes in the lungs where gas exchange occurs.  The transfer of carbon dioxide from the blood into the lungs and of oxygen from the air into the blood takes place in the alveoli.

angiography — The examination of blood vessels by using x-rays after the vessels have been injected with a radiopaque substance.

angiomyolipoma (AML) — A benign tumor (oma) consisting of blood vessels (angio), muscles (myo), and fat (lipo).  AMLs occur in TSC and in LAM and are most often found in the kidneys.
arterial blood gas (ABG) — A measurement of the oxygen and carbon dioxide in the blood obtained by drawing a blood sample from an artery (usually the radial artery near one's wrist or in the brachial artery in the crook of the arm) rather than a vein.  This measurement is considered the most valid for determining the amount of oxygen in the bloodstream.

aspiration — Using a small needle or catheter to suction fluid from a cavity of the body or the inhalation of fluid or a foreign substance, such as vomit, into the bronchi and lungs.

asymptomatic — Presenting no symptoms of disease.

barium — A metallic element.  Barium is mixed with certain fluids and is taken before some x-ray procedures to visually enhance the images.

bilateral transplant — Receiving two lungs during a transplant.  Also called a double-lung transplant.

biopsy — A small sample of tissue taken from an organism for diagnostic purposes.

bleb — A cyst full of fluid and/or air on or near the surface of the lungs.

bronchoalveolar lavage (BAL) — The introduction of a small amount of a sterile saline solution into the lungs during a bronchoscopy to “wash out” tissue samples for study.

bronchitis — A disease characterized by inflammation of the mucous membranes of the bronchial tubes.

bronchodilator — An agent/medicine that relaxes and enlarges the opening of a bronchus or bronchial tube.

bronchodilator response — An expansion of the bronchial tube, after using an agent/medication that allows improved air flow.  An example of a bronchodilator response is when your PFT results improve after you inhale a medication such as Albuterol.

bronchoscopy — A procedure using a flexible fiberoptic tube (bronchoscope) to examine the interior of the lungs.

bronchus pl. bronchi — One of two branches leading out of the trachea and into the lungs.

bulla — A bleb.

capillary — A tiny blood vessel.

cardio-pulmonary exercise test — An exercise test performed on a treadmill or exercycle designed to test exercise capacity by measuring oxygen saturation, pulse, blood pressure, and heart rate.

chest cavity — The space in the body enclosed by the ribs and surrounding the lungs.

chest tubes — Sterile tubes inserted into the chest cavity between the lung and chest wall; used to reinflate the lung, to release air, or to drain chyle or another fluid from the area.

Chronic Obstructive Pulmonary Disease (COPD) — An obstructive lung disease most frequently caused by smoking.  Although LAM is also an obstructive lung disease, most physicians don’t refer to it as COPD.

chyle — A lymphatic fluid that drains from the lymph glands.  It is milky in color and contains fat and other nutrients.

chyloptysis — Expectorating (spitting up/coughing up) chyle.

chylothorax — Chyle in the chest cavity that obstructs the efficiency of the lungs.

chylous ascites — The abnormal accumulation of chyle in the abdominal cavity.

chylous effusion — The abnormal accumulation of chyle in the chest cavity/pleural space.

chyluria — The presence of chyle in the urine stream, giving it a milky appearance.

collapsed lung — A condition where the space between the chest wall and the lung is filled with air or fluid causing the lung to deflate partially or completely.  Also called a pneumo or a pneumothorax.

Computed Tomography Scan (CT or CAT) — An x-ray process where a machine takes pictures of “slices” or planes of the body at specified intervals.  These images give the impression of looking into the body from the top instead of from the front, back, or side.  CT scans look more three-dimensional than standard x-rays.

Cyanosis — A bluish tint or discoloration of the skin and/or mucous membranes due to insufficient oxygenation of the blood.

Diffusing Capacity for Carbon Monoxide (DLCO) — The use of carbon monoxide to measure the diffusion capacity (how well the gasses pass through the membranes) of the lungs.

dyspnea — Shortness of breath (SOB) or difficulty breathing.

echocardiogram — The use of ultrasound to examine the heart.

electrocardiogram (EKG or ECG) —Test  used to measure the activity of the heart muscle.  Electrodes are placed on the chest and extremities, and an electrical current, which passes through the body, is used to detect abnormalities in the heart.

embolization — Therapeutic introduction of various substances into a vessel to occlude or block it. The procedure is used either to arrest or prevent hemorrhaging or to cut off the blood supply to a structure or organ, such that it is  destroyed or weakened.

endotracheal — Inside of or going through the trachea.

etiology — The cause of a disease.

exertional dyspnea — Shortness of breath brought on by exertion.

Expiratory Reserve Volume (ERV) — The extra amount of air you can forcibly exhale after you’ve exhaled normally.

FEV1/FVC — The percent of the vital capacity that can be forcibly expelled in the first second of maximal expiration.

fibroma — Benign tumors consisting primarily of fibrous tissue.

Forced Expiratory Time (FET) — The amount of time (in seconds) the patient exhales during the FVC test.

Forced Expiratory Volume in One Second (FEV1) — The volume of air (in liters) one can forcibly exhale in the first second of maximal expiration. Think: “Blow fast and hard!”

Forced Vital Capacity (FVC) — The total volume of air (in liters) expelled after a full inhalation.

hemoptysis — Coughing or spitting up blood or blood-stained sputum from the lungs or the bronchial tubes.

hypoxemia — Below normal amount of oxygen in arterial blood; lack of sufficient oxygenation of the blood.

hypoxia —Condition in which you and your tissues are oxygen deprived.

Inspiratory Capacity (IC) — The total amount of air that you can draw into your lungs after a normal exhalation.

Inspiratory Reserve Volume (IRV) — The amount of air that you can take in from a normal resting inspiration to the fullest inspiration.

intubation — Placing a tube into the trachea.

leiomyo — Smooth muscle.

leiomyoma — A benign tumor of smooth muscle.  In the uterus, a leiomyoma is known as a fibroid.

leiomyomatosis — The state of having multiple leiomyomas.

lymph — A clear, slightly yellow fluid, containing white blood cells, found in the lymphatic vessels.  Lymph is collected from tissue fluids throughout the body and returned to the blood via the lymphatic system.

Magnetic Resonance Imaging (MRI) — A sophisticated imaging scan which uses magnetism to disrupt cells temporarily and then monitor the energy the cells produce as they return to their normal state.  MRIs are often used for internal structures of the body, especially, but not restricted to, soft tissues and the brain.

metered-dose inhaler — A small, pressurized canister that contains aerosol medicine to be inhaled.  This device releases the exact dose of medicine in small “puffs.”

nebulizer — A device that is pressurized by an oxygen tank and that converts a liquid medicine into a fine mist that can be inhaled.  (This is the device you breathe from during your PFTs when you’re getting the Albuterol.)  Nebulizers are often used in hospitals or when you are unable to use a metered-dose inhaler.

nephrectomy — Removal of a kidney.

obstructive lung disease —Lung disease in which the person experiences limited airflow due to airway obstruction. LAM is an obstructive lung disease.

osteopenia — A decrease in the density of bone mass, often leading to osteoporosis.

osteoporosis — A disease in which the bones lose density and become very porous, and can result in weak and brittle bones.

oxygen (supplemental/therapy) —Supplemental oxygen provides oxygen at concentrations greater than that in room air to raise oxygen levels in the blood. Oxygen delivery systems can be stationary, such as a home concentrator, or portable, such as liquid and canister systems, as well as the newer portable oxygen concentrators (POCs). Oxygen can be received through a nasal cannula or mask.
oxygen saturation (O2 sats) — The percentage of oxygen measured in your blood.  Oxygen saturations are usually measured (totally painlessly) by wearing a small contoured clip called a pulse oximeter on the tip of your finger for a minute or two.  Ideal oxygen saturations are above 90%.

oximeter — See pulse oximeter.

pericardial effusion — An increased amount of fluid in the sac around the heart.

pharynx — The part of the throat that begins from behind the nose to the beginning of the voice box and the esophagus.

pleura — A thin membrane that covers the outside of each lung and folds back to make a lining for the inside of the chest cavity.

pleural abrasion — Roughing up of the pleura by one of various agents to attach the lung to the inside of the chest cavity.  Pleural abrasion is used in attempts to repair and prevent lung collapses (AKA “pneumos”).

pleural effusion — A collection of fluid in the pleural space (chest cavity).

pleurectomy — The removal of the pleura, the membrane enveloping the lungs and lining of the walls of the chest cavity, usually performed to adhere the lungs to the walls of the chest to keep the lungs inflated.

pleurodesis —Procedure to adhere the lining of the lung to the lining of the chest cavity.  The procedure eliminates the space between the lungs and the chest wall and prevents further lung collapses.

pleuroperitoneal shunt — A small tube placed inside the body that connects the chest cavity to the abdominal cavity to drain fluid from the chest.

pneumoretropharynx — Condition that occurs after a pleurodesis when air travels up the airway from the lungs to the back of the throat, causing a "pain in the neck."

pneumonia — An inflammation of the lung usually caused by an infection.

pneumothorax — A lung collapse.  “Pneumo” refers to air and “thorax” to the chest cavity.  In LAM, a pneumothorax occurs when one of the cysts (blebs) bursts and air leaks into the space around the lung.  The pressure of the air between the lung and the chest wall forces the lung to collapse. Sudden shortness of breath, a dry cough, cyanosis (turning blue) and sharp pain felt in the chest, back and/or arms are the main symptoms.

pneumovax —Vaccination to prevent pneumonia.

PO2 or PaO2 — Partial pressure of oxygen in the arterial blood.

PCO or PaCO2 — Partial pressure of carbon dioxide in the arterial blood.

predicted value — The averaged scores of a reference population to which your PFT results are compared.

pulmonary — Pertaining to the lungs.

Pulmonary Function Tests (PFTs) — A series of tests to determine how well the lungs function by checking the performance of the lungs, and used to determine the severity of obstruction and/or restriction in the airways.

pulmonary hemorrhage — Bleeding into or from the lung.

pulmonary rehabilitation — A program of exercise and education to increase pulmonary function and to strengthen the body so it can function more efficiently.

pulse oximeter — A small device that clips onto a fingertip and gives a reading of the oxygen concentration (percentage) in the blood as well as your heart rate at that particular moment.

pursed lip breathing — A breathing technique that can help decrease shortness of breath if performed correctly. Slowly breathe in through your nose. Then pucker your lips as if you were going to kiss someone or whistle, and slowly exhale through your mouth. Try to make your exhalation twice as long as your inhalation. Repeat until your breathlessness eases, or perform whenever you're doing something strenuous.

renal AML — An angiomyolipoma in the kidney.

Residual Volume (RV) — The volume of air remaining in the lungs after maximal exhalation.
respiration — The breathing process that includes exchanges of oxygen and carbon dioxide in the blood.

smooth muscle cell proliferation — The growth and reproduction of smooth muscle cells.  In LAM, these cells take up space meant for healthy lung tissue and form the “blebs” or “cysts” in the lungs.

spacer — A large plastic device with a mouthpiece at one end and a hole at the other end, where the mouthpiece of a metered-dose inhaler is inserted.  A spacer makes it easier to deliver the medication directly into the lungs rather than into the mouth and throat, thus reducing side effects.

spirometer — A computerized machine that measures the volume of air and the speed of exhalation.  
spirometry — A series of tests that are performed using a spirometer and that measure how much (volume) and how fast (flow) you can move air into and out of you lungs.

Sporadic LAM — A type of LAM differentiated from TSC-LAM,  which occurs in conjunction with Tuberous Sclerosis Complex (TSC).  In Sporadic LAM (S-LAM), the genetic mutations occur only in the LAM cells of the organs that are affected, not in the reproductive cells.

sputum — Mucus or phlegm.

talc poudrage — Pleurodesis using medical grade, sterile talc powder which is blown into the pleural space.

talc slurry — Pleurodesis using medical grade, sterile talc liquid that is injected into the pleural space.

thoracentesis — The passage of a hollow tube or needle into the chest cavity to draw off fluid.

thoracostomy — A surgical opening of the chest (as for drainage).

thoracotomy — Open-chest surgery; an incision into the chest wall for a biopsy or for a procedure such as pleurodesis. VATS is a much less invasive alternative.

thorascopic surgery — Surgery performed inside the chest cavity using a thorascope (an optical instrument with a lighted tip) to assist the surgery.  The technique is also called VATS (video-assisted thoracic surgery).

thorax — The part of the body that is situated between the neck and the abdomen and that is supported by the ribs, costal cartilages, sternum, and dorsal spine.

Tidal Volume (TV) — The amount of air you inhale and exhale during your resting or normal breathing pattern.

Total Lung Capacity (TLC) — The total volume of air in the lungs after a maximal inspiration.

trachea — The tube connecting your mouth and nose to your lungs; windpipe.

transplant — The transfer of an organ (or tissue) from one body to another.

TS- or TSC-LAM — LAM that occurs in conjunction with Tuberous Sclerosis Complex.
Tuberous Sclerosis (TS) or Tuberous Sclerosis Complex (TSC) — A genetic disorder characterized by tumors of the brain, heart, kidney, lungs and skin; seizures; and mental retardation.  Not all individuals with TSC will have all of these symptoms.

ultrasonography — See ultrasound.

ultrasound — A noninvasive method of viewing internal organs using sound waves.  A handheld “microphone” is moved over the area of an organ to detect abnormalities.

unilateral transplant — A single-lung transplant.

Video-assisted thoracic surgery (VATS) — Surgery using an optical instrument with a lighted tip.  (See thorascopic surgery.)

Vital Capacity (VC) — The amount of air that you can forcibly exhale after you have fully inhaled.