Cystic Fibrosis Quiz

Questions and Answers

What is the defect in the gene coding for Cystic Fibrosis Transmembrane Regulator (CFTR)?

Defect in chloride channel function (correct)

Defect in potassium channel function

Defect in calcium channel function

Defect in sodium channel function

What is the commonest autosomal recessive condition?

Sickle cell anemia

Tay-Sachs disease

Hemophilia

Cystic Fibrosis (correct)

What is the minimum sweat volume required for diagnosis of Cystic Fibrosis?

Not less than 75µl (correct)

Not less than 100µl

Not less than 25µl

Not less than 50µl

What is the sweat chloride concentration indicative of Cystic Fibrosis?

Sweat chloride concentration > 60mmol/l Signup and view all the answers

Which infection is successfully treated with 3 weeks of oral ciprofloxacin and 3 months of nebulised colomycin at age 7?

1st Pseudomonas aeruginosa infection Signup and view all the answers

At what age did the individual experience a recurrence of Pseudomonas infection?

10 and 12 Signup and view all the answers

What is the age at which Allergic Bronchopulmonary Aspergillosis (ABPA) was diagnosed?

12 Signup and view all the answers

What is the age at which the individual had a Mycobacterium Kansasii infection?

10 Signup and view all the answers

What is the age at which the individual had a successful eradication of Pseudomonas infection with IV therapy?

9 Signup and view all the answers

What is the age at which the individual had a Pilocarpine iontophoresis technique done?

Not mentioned Signup and view all the answers

What is the age at which the individual had newborn screening for Cystic Fibrosis?

5/6 days Signup and view all the answers

Which structure forms from the inner cell mass during implantation?

Epiblast Signup and view all the answers

When does gastrulation, resulting in the formation of three germ layers, occur?

Day 17 Signup and view all the answers

What is the outcome of the divisions of the zygote?

Blastocyst Signup and view all the answers

Where does the zygote implant during the process of implantation?

Mother's uterus Signup and view all the answers

What are the two layers of cells that form from the inner cell mass during implantation?

Epiblast and hypoblast Signup and view all the answers

What is the most common type of congenital diaphragmatic hernia?

Postero-lateral (Bochdalek) Signup and view all the answers

What is the prevalence of oesophageal atresia and tracheoesophageal fistula among births?

1 in 3000 Signup and view all the answers

What is the main cause of respiratory distress syndrome in newborns?

Premature birth and insufficient surfactant Signup and view all the answers

What is the prevalence of congenital pulmonary airway malformation among births?

1 in 30,000 Signup and view all the answers

At what stage of lung development do divisions of the alveoli occur, which continues after birth?

Alveolar Signup and view all the answers

Which structure separates the thoracic and abdominopelvic cavities during embryonic development?

Diaphragm Signup and view all the answers

Which phase of lung development involves generational divisions to create the bronchial tree?

Pseudoglandular Signup and view all the answers

What contributes to the development of the larynx during embryonic development?

Pharyngeal arches 4 and 6 Signup and view all the answers

What separates the trachea and esophagus during embryonic development?

Tracheoesophageal septum Signup and view all the answers

What develops as an outpouching of the foregut during lung development?

Respiratory diverticulum (lung bud) Signup and view all the answers

Study Notes

Embryonic Development of Body Cavities and Lungs

Mesoderm develops into paraxial, intermediate, and lateral plate mesoderm
Lateral plate mesoderm divides into parietal and visceral layers by the development of intraembryonic coelom
Lateral folding of the embryonic "disc" causes the closing of body walls, entrapping intraembryonic coelom into a closed body cavity
The continuous primitive body cavities undergo partitioning as lung buds invaginate into pericardioperitoneal canals
The diaphragm forms to separate thoracic and abdominopelvic cavities
The diaphragm is composed of septum transversum, pleuroperitoneal membranes, mesentery of the esophagus, and the body wall
The respiratory diverticulum (lung bud) develops as an outpouching of the foregut, with endoderm and visceral lateral plate mesoderm origin
Tracheoesophageal ridges fuse to form the tracheoesophageal septum, separating the trachea and esophagus
Pharyngeal arches 4 and 6 contribute to the development of the larynx
The internal epithelium of the larynx is from the endoderm, while laryngeal muscles and cartilages come from the fusing of mesenchyme from arches 4 and 6
Lung development includes generational divisions to create the bronchial tree, with four phases of development
Lung development includes four phases: pseudoglandular, canalicular, saccular, and alveolar, with each phase having specific characteristics and timeframes

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Description

Test your knowledge of Cystic Fibrosis with this quiz covering its pathophysiology, diagnosis, standard care, and future implications. Explore the commonest autosomal recessive condition, CFTR gene defects, mutations, and associated symptoms.