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Glossary for Pulmonary hypertension

  • Abdominal swelling: Swelling or bloating of the abdomen
  • Absence of pulmonary artery: The absence of a pulmonary artery at birth.
  • Acid-Base Imbalance: A disruption to the normal acid-base equilibrium in the body. There are four main groups of disorder involving an acid-base imbalance: respiratory acidosis or alkalosis and metabolic acidosis or alkalosis. Obviously the severity of symptoms is determined by the degree of imbalance.
  • Adult Cystic Fibrosis: Cystic fibrosis is an inherited condition characterized by the production of thick sticky mucus by the mucus glands in the lungs, intestines, liver and pancreas. The condition is most often diagnosed in children or young adults but occasionally, relatively mild symptoms may lead to frequent misdiagnosis or no diagnosis at all unless the symptoms become worse. The condition may be misdiagnosed as emphysema, asthma or chronic bronchitis. It is usually females with a mild form of the disease who tend to be diagnosed at a later age.
  • Alveolar capillary dysplasia: The abnormal development of the lung blood vessels. The normal barrier across which air and blood can diffuse fails to develop properly. Death usually results within weeks of birth but rare cases can survive for months.
  • Ankle swelling: Ankle swelling often from injury or fluid retention.
  • Anoxia: Lack of oxygen to the body's tissues.
  • Artery conditions: Any conditions affecting arteries
  • Asthma in Adults:
  • Asthma in Children:
  • Avasthey syndrome: A very rare syndrome characterized by pulmonary hypertension, lymphedema and malformation of brain blood vessels.
  • Blood conditions: Conditions that affect the blood
  • Bone cement implantation syndrome: A complication that can occur after using bone cement during implantation of an internal prosthesis. Circulation may be affected and a blockage in the pulmonary artery may develop.
  • Bronchiectasis: Chronic bronchiole dilation from secretions and blockages.
  • COPD: Severe obstruction of bronchial air flow typically from bronchitis and/or emphysema.
  • Chest pain: Pain in the chest area.
  • Chronic cough: The chronic noisy sudden expulsion of air from the respiratory tract
  • Churg-Strauss Syndrome: A condition characterized by blood vessel inflammation that occurs throughout the body.
  • Coal worker's pneumoconiosis: A lung disease caused by breathing in coal dust. The condition is generally associated with employees in the coal industry. Symptoms usually resolve when exposure to coal dust stops.
  • Congenital heart defects: Heart defects that a baby is born with.
  • Congenital mitral malformation: Malformations of the mitral valve that are present at birth. The mitral valve allows blood to flow between the two chambers on the left side of the heart. Types of mitral malformation are atresia (absent mitral valve), stenosis (narrowed mitral valve) and parachute mitral valve.
  • Congenital mitral stenosis: A heart malformation that is present at birth. The mitral valve is narrower than normal which affects blood flow between the upper and lower chambers on the left side of the heart. It usually occurs in conjunction with other malformations
  • Congestive Heart Failure: Inadequate pumping and decline of heart function common in the elderly.
  • Convulsions: Involuntary spasms especially those affecting the full body
  • Cor Triatriatum: A rare congenital malformation where the heart has three atria instead of the normal two due to the presence of a separating membrane.
  • Cor pulmonale: Enlarged heart due to respiratory difficulty.
  • Cough: The noise produced from the sudden expulsion of air from the lungs
  • Cystic Fibrosis: Cystic fibrosis is a hereditary disease affecting the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.
  • Diffuse systemic sclerosi: A rare condition that characterized by skin tightness affecting the trunk and extremities as well as organ involvement. It involves an early period of internal inflammation which distinguishes it from other forms of scleroderma and the organ involvement can be severe and even life-threatening.
  • Down Syndrome: A chromosome syndrome causing physical effects and mental retardation.
  • Eisenmenger Syndrome: Increased lung blood pressure that can result from conditions such as a hole in the wall between the two heart chambers.
  • Emphysema: Emphysema is a type of chronic obstructive pulmonary disease (COPD). It is defined as an abnormal, permanent enlargement of the air spaces distal to the terminal bronchioles accompanied by destruction of their walls and without obvious fibrosis.
  • Gaucher Disease: A rare inherited biochemical disorder characterized by the deficiency of the enzyme called glucocerebrosidase and accumulation of glycosylceramide (glucocerebroside). There are three forms of this disease: type 1, 2 and 3.
  • Gaucher disease type 3: A rare inherited biochemical disorder characterized by the deficiency of the enzyme called glucocerebrosidase and accumulation of glycosylceramide (glucocerebroside). There are three forms of this disease: type 1, 2 and 3. Type 3 is a subacute neurological form which often first appears in childhood.
  • Glycine encephalopathy, classical neonatal early-onset form: A rare disorder of amino acid metabolism where glycine are unable to be metabolized properly due to defects in the glycine cleavage system. The early onset classical neonatal form usually starts after a period of normal development during the first 6 months of life.
  • Glycine encephalopathy, classical neonatal form: A rare disorder of amino acid metabolism where glycine are unable to be metabolized properly due to defects in the glycine cleavage system.
  • Glycine encephalopathy, classical neonatal late-onset form: A rare disorder of amino acid metabolism where glycine are unable to be metabolized properly due to defects in the glycine cleavage system. The late onset classical neonatal form usually starts during childhood.
  • Glycine synthase deficiency: A rare genetic disorder characterized by high blood glycine levels which is toxic to the body. The severity of the condition varies according to the degree of deficiency and age of onset. The classical neonatal form is generally quite severe, the atypical mild form which generally includes symptoms such as aggressiveness, behavioral problems and speech problems. The transient neonatal form involves high blood glycine levels at birth which then returns to normal within a couple of months - there was no neurological or developmental impairment.
  • Glycine synthase deficiency, type 1: A rare genetic disorder characterized by high blood glycine levels. It is caused by a defect in the P protein (pyridoxal phosphate-dependent glycine decarboxylase) in the energy creating center of cells (mitochondria).
  • Glycine synthase deficiency, type 2: A rare genetic disorder characterized by high blood glycine levels. It is caused by a defect in the T protein (tetrahydrofolate-requiring enzyme) in the energy creating center of cells (mitochondria).
  • Heart failure: Slow failure of the heart (cardiac insufficiency).
  • Heart symptoms: Symptoms affecting the heart
  • Hemangiomatosis, familial pulmonary capillary: A rare condition involving the excessive proliferation of lung capillaries which results in pulmonary hypertension.
  • Hypereosinophilic syndrome: A rare condition where too many eosinophils are produced over an extended period of time for no apparent reason. The eosinophils can infiltrate various organs and tissues and cause dysfunction or damage
  • Hypertension: High blood pressure
  • Idiopathic pulmonary hypertension: A rare condition where sclerosis of the pulmonary arteries cause cyanosis, polycythemia and heart failure.
  • Interstitial lung disease: Any condition which affects the interstitium of the lungs
  • LEOPARD Syndrome: A rare genetic disorder characterized by multiple lentigines, pulmonic stenosis, widely spaced eyes and deafness.
  • Limited cutaneous systemic sclerosis: A rare disorder that primarily affects the skin and blood vessels. It is characterized by calcified skin deposits (C), Raynaud syndrome (R), esophageal dysfunction (E), scleroderma of the fingers and toes(S) and telengiectasia - dilated blood vessels (T).
  • Lung conditions: Various conditions affecting the lungs or related airways.
  • Lupus: Autoimmune disease with numerous effects on various organs and linings.
  • Lymphangiomatosis, pulmonary: A rare disorder characterized by the presence of numerous small lung cysts at birth which severely affects breathing and blood pressure and generally results in infant death.
  • Methylcobalamin deficiency cbl G type: An inherited organic acid disorder where an enzyme deficiency (cbl G) impairs the body's ability to break down certain proteins consumed in the diet. This results in a buildup of homocystine which results in harmful affects.
  • Mitral stenosis: Narrowing of the mitral heart valve
  • Obstructive sleep apnea: Obstructive sleep apnea (OSA) syndrome is characterized by episodic upper airway obstruction that occurs during sleep.
  • Pickwickian syndrome: A syndrome characterized by obesity, somnolence, hypoventilation and erythrocytosis
  • Pierre Robin's sequence: A rare genetic disorder characterized by an underdeveloped jaw, cleft soft palate and abnormal tongue location.
  • Portal hypertension: Portal hypertension may be defined as a portal pressure gradient of 12 mm Hg or greater and is often associated with varices and ascites.
  • Possible human carcinogenic exposure - Monocrotaline: Some evidence indicates that exposure to Monocrotaline has a possible link to an increased risk of developing cancer in humans. The carcinogenicity of the substance may be influenced by the duration and level of exposure. Monocrotaline is a plant toxin which can be found as a contaminant in grains or in some herbal remedies.
  • Primary pulmonary hypertension: Primary pulmonary hypertension refers to high blood pressure in the arteries that carry blood to the lungs for no apparent reason. Blood pressure in other parts of the body is normal or sometimes even low.
  • Pulmonary alveolar microlithiasis: A rare form of lung disease characterized by tiny deposits of calcium phosphate throughout the alveoli in the lungs. The abnormal depositions may start at any age and the condition may progress very slowly with no symptoms for years or even decades. Cases which involve rapid progression or early onset are very uncommon.
  • Pulmonary artery agenesis: A rare defect where the pulmonary artery or a portion of it fails to develop. The pulmonary artery has two branches, each of which goes to a different lung. The affected lung tends to be smaller and the bronchial arteries that supply it tend to be enlarged.
  • Pulmonary cystic lymphangiectasis: A rare congenital condition where the lungs fail to develop normally. The disorder is characterized by the presence of dilated lymph ducts throughout the lungs.
  • Pulmonary embolism: Blocked lung blood vessel often from a blood clot.
  • Pulmonary fibrosis: A scarring condition that affects the lungs
  • Pulmonary fibrosis /granuloma: A lung disease characterized by stiffening and inflammation of lung tissue.
  • Pulmonary veno-occlusive disease: Any disease which causes a lung vein to become narrow or blocked. The pulmonary vein carries oxygenated blood from the lungs to various body tissues.
  • Pulmonary venous return anomaly: A rare disorder where one or more of the four veins that carry oxygenated blood from the lungs drain to the right atrium of the heart instead of the left atrium. Symptom severity is determined by the number of veins involved and the exact location of the heart that the veins drain into.
  • Reduced heart function: A reduction from normal of heart function
  • Respiratory acidosis: Respiratory acidosis is acidosis (abnormally increased acidity of the blood) due to decreased ventilation of the pulmonary alveoli, leading to elevated arterial carbon dioxide concentration.
  • Respiratory conditions: Any condition that affects the respiratory system
  • Respiratory failure: Failure of the respiratory system
  • Right heart failure: Heart failure of the right side of the heart
  • Scleroderma: A rare, progressive connective tissue disorder involving thickening and hardening of the skin and connective tissue. There are a number of forms of scleroderma with some forms being systemic (involving internal organs).
  • Secondary pulmonary hypertension: Secondary pulmonary hypertension refers to high blood pressure in the arteries that carry blood to the lungs. Blood pressure in other parts of the body is normal or sometimes even low. The condition may be caused by such things as lung conditions (e.g. emphysema, chronic bronchitis), heart conditions (e.g. congestive heart failure, birth defects involving heart), AIDS or medications such as fenfluramine (a diet drug).
  • Shortness of breath: The feeling of being short of breath
  • Silicosis: An occupation lung disease caused by breathing in silica dust.
  • Sleep apnea: Childhood obstructive sleep apnea (OSA) syndrome is characterized by episodic upper airway obstruction that occurs during sleep.
  • Takayasu arteritis: A rare disorder involving inflammation of large elastic arteries including the aorta which impairs blood flow to the upper body.
  • Unilateral pulmonary agenesis: A very rare birth defect where one side of the lung is incompletely developed or totally absent. The symptoms and severity of the condition is variable and other malformation are often associated with the condition.
  • Vater-like syndrome, with pulmonary hypertension, abnormal ears and growth deficiency: A rare syndrome characterized by unusual ears, retarded growth, pulmonary hypertension.
  • Ventricular septal defect: An abnormal connection between the 2 lower chambers (ventricles) of the heart.
  • Wolman disease: A rare inherited lipid storage disease. The condition usually results in death early in life.


 » Next page: Clinical Trials for Pulmonary hypertension

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