Biomaterial Database

Guidance of children and adolescents with cystic fibrosis. 1990

J Gerritsen, and H van der Laag, and G Sinnema, and K Knol

University Hospital, Groningen, The Netherlands.

Cystic fibrosis is the most common serious genetic disorder in people of European descent. Treatment of these patients is ongoing throughout life and until now has been aimed at the consequences and is still not curative. Over the past 10-20 years, there has been a dramatic improvement of mortality rates for cystic fibrosis, due in large part to advances in medical care. The average age of survival for young people with cystic fibrosis is pushing well into the 20s with one third living into their 30s. Consequently, education plays a major role in management of patients with cystic fibrosis, and starts directly after being sure of the diagnosis. Growing up, these patients experience a lot of problems, and these are especially marked in the adolescent. A special problem, for many cystic fibrosis patients is becoming an adult. Continuity in care for these patients from the pediatric to the adult department is not always guaranteed. It is concluded that patients with cystic fibrosis should be treated in specialized centers, and such treatment cannot be carried out sufficiently by one person, but has to be embedded in a team of caregivers.

UI	MeSH Term	Description	Entries
D010353	Patient Education as Topic	The teaching or training of patients concerning their own health needs.	Education of Patients,Education, Patient,Patient Education
D011788	Quality of Life	A generic concept reflecting concern with the modification and enhancement of life attributes, e.g., physical, political, moral, social environment as well as health and disease.	HRQOL,Health-Related Quality Of Life,Life Quality,Health Related Quality Of Life
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003131	Combined Modality Therapy	The treatment of a disease or condition by several different means simultaneously or sequentially. Chemoimmunotherapy, RADIOIMMUNOTHERAPY, chemoradiotherapy, cryochemotherapy, and SALVAGE THERAPY are seen most frequently, but their combinations with each other and surgery are also used.	Multimodal Treatment,Therapy, Combined Modality,Combined Modality Therapies,Modality Therapies, Combined,Modality Therapy, Combined,Multimodal Treatments,Therapies, Combined Modality,Treatment, Multimodal,Treatments, Multimodal
D003550	Cystic Fibrosis	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.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293	Adolescent	A person 13 to 18 years of age.	Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D012803	Sick Role	Set of expectations that exempt persons from responsibility for their illness and exempt them from usual responsibilities.	Role, Sick,Roles, Sick,Sick Roles