C0010674 - A congenital, autosomal, metabolic disorder affecting the exocrine glands. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production that causes obstruction of passageways, including pancreatic and bile ducts, intestines, and bronchi. Symptoms usually appear in childhood, and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather secondary to increased sodium and chloride concentration in sweat.

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Lang Dictionary CODE			LUI	preferred no	SUI	preference Yes / No	Terms, descriptions
							CUI C0010674

Russian	Medical Subject Headings Russian	D003550	L3352209	preferred	S3879693	Y	КИСТОЗНЫЙ ФИБРОЗ
Russian	Medical Subject Headings Russian	D003550	L0896959	no	S1100776	Y	KISTOZNYI FIBROZ
Russian	Medical Subject Headings Russian	D003550	L1519801	no	S1815710	Y	FIBROZNO-KISTOZNAIA BOLEZN' PODZHELUDOCHNOI ZHELEZY
Russian	Medical Subject Headings Russian	D003550	L1531283	no	S1827192	Y	MUKOVISTSIDOZ
Russian	Medical Subject Headings Russian	D003550	L1535323	no	S1831232	Y	PANKREATICHESKII KISTOZNYI FIBROZ
Russian	Medical Subject Headings Russian	D003550	L1535329	no	S1831238	Y	PANKREOFIBROZ
Russian	MDRRUS	10028141	L15736866	no	S19047825	N	Муковисцидоз
Russian	MDRRUS	10011762	L15736866	no	S19047825	N	Муковисцидоз
Russian	MDRRUS	10011762	L15736866	no	S19047825	Y	Муковисцидоз
Russian	MDRRUS	10011764	L16135087	no	S19405033	Y	Муковисцидоз, БДУ
Russian	Medical Subject Headings Russian	D003550	L3358788	no	S3886274	Y	МУКОВИСЦИДОЗ
Russian	Medical Subject Headings Russian	D003550	L3362206	no	S3889680	Y	ПАНКРЕАТИЧЕСКИЙ КИСТОЗНЫЙ ФИБРОЗ
Russian	Medical Subject Headings Russian	D003550	L3362219	no	S3889693	Y	ПАНКРЕОФИБРОЗ
Russian	Medical Subject Headings Russian	D003550	L3374181	no	S3901670	Y	ФИБРОЗНО-КИСТОЗНАЯ БОЛЕЗНЬ ПОДЖЕЛУДОЧНОЙ ЖЕЛЕЗЫ
	Medical Subject Headings				A0044709	AT43116146	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.
	(CPTSP) CRISP Thesaurus				A0477116	AT51220204	most common potentially lethal autosomal recessive disease affecting Caucasians; characterized by chronic pulmonary, intestinal, liver, pancreatic, and exocrine gland dysfunction; caused by mutations of the CFTR chloride channel.
	Medical Subject Headings Czech				A13066282	AT204247608	Zkr. CF, mukoviscidóza - autozomálně recesivně dědičné, relativně časté onemocnění žláz se zevní sekrecí. Problémem je defektní transport iontů zvyšující viskozitu sekretů exokrinních žláz. Mutován je gen pro CFTR na 7. chromozomu. Nápadná je vysoká koncentrace chloridů v potu (lze využít i diagnosticky, tzv. potní test). Porucha jejich funkce se projevuje zejm. v plicích (opakované záněty při ucpávání průdušek hleny, porucha dýchání, respirační insuficience), v trávicím ústrojí (porucha trávení při nedostatečnosti slinivky břišní). Bývá sterilita či snížená fertilita, vyšší je rovněž výskyt diabetu (srov. CFRD). Navazují i příznaky podmíněné malabsorpcí. Některé z těchto příznaků vystupují do popředí s prodlužujícím se životem postižených. V terapii jsou důležitá antibiotika, péče o drenáž dýchacích cest, symptomatická terapie trávicích obtíží, péče o výživu, ev. protizánětlivé léky; z moderních postupů transplantace plic a v budoucnosti genová terapie. Projevuje se od raného dětství, dř. se děti nedožily puberty, v dnešní době se s intenzivní terapií většina dožívá dospělosti. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
	MEDLINEPLUS				A21145367	AT220105872	Cystic fibrosis (CF) is an inherited disease of the mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs. CF causes your mucus to be thick and sticky. The mucus clogs the lungs, causing breathing problems and making it easy for bacteria to grow. This can lead to repeated lung infections and lung damage. The symptoms and severity of CF can vary. Some people have serious problems from birth. Others have a milder version of the disease that doesn't show up until they are teens or young adults. Sometimes you will have few symptoms, but later you may have more symptoms. CF is diagnosed through various tests, such as gene, blood, and sweat tests. There is no cure for CF, but treatments have improved greatly in recent years. In the past, most deaths from CF were in children and teenagers. Today, with improved treatments, some people who have CF are living into their forties, fifties, or older. Treatments may include chest physical therapy, nutritional and respiratory therapies, medicines, and exercise. NIH: National Heart, Lung, and Blood Institute
	NCI NCI Dictionary of Cancer Terms				A7569881	AT197992364	A common hereditary disease in which exocrine (secretory) glands produce abnormally thick mucus. This mucus can cause problems in digestion, breathing, and body cooling.
	NCI National Institute of Child Health and Human Development				A7569881	AT210369871	A congenital, autosomal, metabolic disorder affecting the exocrine glands. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production that causes obstruction of passageways, including pancreatic and bile ducts, intestines, and bronchi. Symptoms usually appear in childhood, and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather secondary to increased sodium and chloride concentration in sweat.
	NCI Thesaurus				A7569881	AT210371170	A congenital, autosomal, metabolic disorder affecting the exocrine glands. The secretions of exocrine glands are abnormal, resulting in excessively viscid mucus production that causes obstruction of passageways, including pancreatic and bile ducts, intestines, and bronchi. Symptoms usually appear in childhood, and include meconium ileus, poor growth despite good appetite, malabsorption and foul bulky stools, chronic bronchitis with cough, recurrent pneumonia, bronchiectasis, emphysema, clubbing of the fingers, and salt depletion in hot weather secondary to increased sodium and chloride concentration in sweat.