THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
Bronchiectasis
A condition characterized by chronic permanent dilation & destruction of bronchi due to destructive changes in the elastic and muscular layers of bronchial walls.
The common thread in the pathogenesis of bronchiectasis consists of difficulty clearing secretions & recurrent infections with a “vicious circle” of infection and inflammation resulting in airway injury and remodelling.
PLEASE REFER TO REFERENCE TEXTBOOKS FOR CLARITY.
Atelectasis is a complete or partial collapse of the entire lung or area (lobe) of the lung. It occurs when the tiny air sacs (alveoli) within the lung become deflated or possibly filled with alveolar fluid.
Cystic fibrosis (also known as CF or mucoviscidosis) is a recessive multi-system genetic disease characterized by abnormal transport of chloride and sodium across epithelium, leading to thick, viscous secretions in the lungs, pancreas, liver, intestine.[Yankas JR, et al. (2004). "Cystic fibrosis adult care consensus conference report". Chest 125: 1-39.]
Bronchiectasis
A condition characterized by chronic permanent dilation & destruction of bronchi due to destructive changes in the elastic and muscular layers of bronchial walls.
The common thread in the pathogenesis of bronchiectasis consists of difficulty clearing secretions & recurrent infections with a “vicious circle” of infection and inflammation resulting in airway injury and remodelling.
PLEASE REFER TO REFERENCE TEXTBOOKS FOR CLARITY.
Atelectasis is a complete or partial collapse of the entire lung or area (lobe) of the lung. It occurs when the tiny air sacs (alveoli) within the lung become deflated or possibly filled with alveolar fluid.
Cystic fibrosis (also known as CF or mucoviscidosis) is a recessive multi-system genetic disease characterized by abnormal transport of chloride and sodium across epithelium, leading to thick, viscous secretions in the lungs, pancreas, liver, intestine.[Yankas JR, et al. (2004). "Cystic fibrosis adult care consensus conference report". Chest 125: 1-39.]
Pleural effusion may be defined figuratively as the juice, oozing from the leaky lingerie of the lung. However the text book definition is the abnormal accumulation of fluid in the pleural space due to disturbances in the forces that keep the pleural fluid economy in equilibrium...
Apparently a lengthy presentation actually very good for junior physicians as it covers all aspects of assessment, diagnosis and treatment of pleural effusion
CYSTIC FIBROSIS AND PHYSIOTHERAPY TREATMENT.pptxShilpasree Saha
Physiotherapy has long been considered a cornerstone of condition management for
people with cystic fibrosis (CF). The presentation of CF has changed over time with an increased
life expectancy and increased expectations of people with CF to have a complete lifestyle.
abscess advanced trauma life support anterior open bite antibiotics braces csf leaks dental diseases doxycycline dr dr shabeel drshabeel’s face eye trauma gingival infection medical medicine periodontal gum surgery pharmacy pn
Mistry Shivangi, M.pharm in Pharmacology, Assistant Professor in BMCP, Definition, Difference between COPD and Asthma, Causes, Pathophysiology, symptoms, Complication, Diagnosis, Treatment and Pro-diagnosis
Weekly endorsement during our rotation in the Department of Family and Community Medicine. This is the case of a 72-year-old male who came in due to difficulty of breathing
The Indian economy is classified into different sectors to simplify the analysis and understanding of economic activities. For Class 10, it's essential to grasp the sectors of the Indian economy, understand their characteristics, and recognize their importance. This guide will provide detailed notes on the Sectors of the Indian Economy Class 10, using specific long-tail keywords to enhance comprehension.
For more information, visit-www.vavaclasses.com
We all have good and bad thoughts from time to time and situation to situation. We are bombarded daily with spiraling thoughts(both negative and positive) creating all-consuming feel , making us difficult to manage with associated suffering. Good thoughts are like our Mob Signal (Positive thought) amidst noise(negative thought) in the atmosphere. Negative thoughts like noise outweigh positive thoughts. These thoughts often create unwanted confusion, trouble, stress and frustration in our mind as well as chaos in our physical world. Negative thoughts are also known as “distorted thinking”.
The French Revolution, which began in 1789, was a period of radical social and political upheaval in France. It marked the decline of absolute monarchies, the rise of secular and democratic republics, and the eventual rise of Napoleon Bonaparte. This revolutionary period is crucial in understanding the transition from feudalism to modernity in Europe.
For more information, visit-www.vavaclasses.com
Instructions for Submissions thorugh G- Classroom.pptxJheel Barad
This presentation provides a briefing on how to upload submissions and documents in Google Classroom. It was prepared as part of an orientation for new Sainik School in-service teacher trainees. As a training officer, my goal is to ensure that you are comfortable and proficient with this essential tool for managing assignments and fostering student engagement.
Welcome to TechSoup New Member Orientation and Q&A (May 2024).pdfTechSoup
In this webinar you will learn how your organization can access TechSoup's wide variety of product discount and donation programs. From hardware to software, we'll give you a tour of the tools available to help your nonprofit with productivity, collaboration, financial management, donor tracking, security, and more.
Read| The latest issue of The Challenger is here! We are thrilled to announce that our school paper has qualified for the NATIONAL SCHOOLS PRESS CONFERENCE (NSPC) 2024. Thank you for your unwavering support and trust. Dive into the stories that made us stand out!
The Roman Empire A Historical Colossus.pdfkaushalkr1407
The Roman Empire, a vast and enduring power, stands as one of history's most remarkable civilizations, leaving an indelible imprint on the world. It emerged from the Roman Republic, transitioning into an imperial powerhouse under the leadership of Augustus Caesar in 27 BCE. This transformation marked the beginning of an era defined by unprecedented territorial expansion, architectural marvels, and profound cultural influence.
The empire's roots lie in the city of Rome, founded, according to legend, by Romulus in 753 BCE. Over centuries, Rome evolved from a small settlement to a formidable republic, characterized by a complex political system with elected officials and checks on power. However, internal strife, class conflicts, and military ambitions paved the way for the end of the Republic. Julius Caesar’s dictatorship and subsequent assassination in 44 BCE created a power vacuum, leading to a civil war. Octavian, later Augustus, emerged victorious, heralding the Roman Empire’s birth.
Under Augustus, the empire experienced the Pax Romana, a 200-year period of relative peace and stability. Augustus reformed the military, established efficient administrative systems, and initiated grand construction projects. The empire's borders expanded, encompassing territories from Britain to Egypt and from Spain to the Euphrates. Roman legions, renowned for their discipline and engineering prowess, secured and maintained these vast territories, building roads, fortifications, and cities that facilitated control and integration.
The Roman Empire’s society was hierarchical, with a rigid class system. At the top were the patricians, wealthy elites who held significant political power. Below them were the plebeians, free citizens with limited political influence, and the vast numbers of slaves who formed the backbone of the economy. The family unit was central, governed by the paterfamilias, the male head who held absolute authority.
Culturally, the Romans were eclectic, absorbing and adapting elements from the civilizations they encountered, particularly the Greeks. Roman art, literature, and philosophy reflected this synthesis, creating a rich cultural tapestry. Latin, the Roman language, became the lingua franca of the Western world, influencing numerous modern languages.
Roman architecture and engineering achievements were monumental. They perfected the arch, vault, and dome, constructing enduring structures like the Colosseum, Pantheon, and aqueducts. These engineering marvels not only showcased Roman ingenuity but also served practical purposes, from public entertainment to water supply.
1. Cystic Fibrosis:
“Cystic fibrosis is an inherited
multisystem disorder Characterized by
obstruction & infection of airways &
maldigestion & its consequences”.
2. Genetics:
AR – 1/3500 live births
Gene locus on long arm of chromosome 7
CF gene -CF transmembrane regulator(CFTR)
CFTR- cAMP - regulated chloride channel
Expressed on ephitheial cells
>1500 mutations-most prevalent ∆F508
3. The net ion flow across normal and cystic fibrosis cell
4. Pathophysiology (cont…)
1- Paucity of water in mucus secretions
2- Failure to clear mucous secretions
3- Ch. infection limited to respiratory tract
4- Elevated salt content of sweat & other
serous secretions
7. Pancreas:
Small & cystic
In 85-90% of cases replacement with fibrous
tissue & fat
Foci of calcification may occur
Islets of langerhans – architectural disruption
11. Clinical Manifestations:
Respiratory Tract:
Symptoms
Cough, Wheezing, Shortness of breath
Exercise intolerance
Rhinorhea, Nasal obstruction
Signs
Cyanosis, Digital clubbing,
Increased AP diameter of chest
Gen.hyper resonance, Coarse crackles,
Expiratory wheeze
Acute sinusitis, Nasal polyps
12. Intestinal Tract:
Meconium Ileus:
In 15-20% Newborn
Meconium Plug Syndrome:
Less specific than meconium ileus
Distal intestinal obstruction
with fecal material in older pts
13. Intestinal Tract:
– Symptoms
Greasy stool, Excessive flatus
Acid or bile reflux
Vit. Deficiencies- ADEK
– Signs
Protuberant abdomen
Intussusception
Fecal impaction of cecum
Subacute appendicitis,
Anasarca
16. Genitourinary system:
An average of 2 yrs delay in sexual development
Males
>95% azoospermic
Sexual function unimpaired
Female
Cervicitis
Secondary amenorrhea
Decrease fertility
Pregnancy well tolerated
18. Diagnostic Criteria for Cystic Fibrosis
Presence of typical clinical features
OR
History of CF in a sibling
OR
Positive newborn screening test
PLUS
Lab evidence for CFTR dysfunction
Two elevated sweat Chloride tests
OR
Identification of 2 CF mutations
OR
An abnormal nasal potential difference
measurement
19. Diagnosis:
Sweat Testing
> 60mEq/L of chloride in sweat
Threshold levels of 40mEq/L have
been suggested
Values b/w 40-60mEq/L suggest
CF at all ages
20. Radiology
Pulmonary
Hyperinflation of lung,
Atelactasis & Bronchiectasis
Dilated pulmonary artery segment
Paranasal Sinuses
Pan opacification
Failure of frontal sinus to develop
Fetal USG
Ileal obstruction with meconium in 2nd
trimester
21. Pulmonary Function
Not obtained till 5-6 yrs of age
RV & FRC are increased (early)
Declining total lung capacity & VC(late)
Microbiological Studies
Staph aureus,
pseudomonas aeruginosa, B. cepacia
22. Newborn Screening
Immunoreactive trysinogen in blood spots
Confirmatory sweat & DNA testing
Other Investigations as Indicated
Cardiac: ECG, Echocardiography
GIT: S. proteins, LFTs, coagulation
studies, x-ray abdomen, USG abdomen,
hepatobiliary Tc-99 Scintigraphy, PTC,
ERCP, pancreatic function tests
Other: urea & electrolytes
23. Management of CF:
Aims:
1.
2.
3.
4.
5.
6.
Ensure optimal growth & development
Delaying progress of pulmonary disease
Preventing & treating complications
Normal life style
Patient & family education
Recognition & treatment of psychological
complications
Multidisciplinary approach
24. Treatment:
1- Treatment of Lung disease
2- Treatment of Gastrointestinal disease
3- Nutritional therapy
4- Treatment of Complications
29. Bronchodilator Treatment:
Increased bronchial activity in 40-75 % pts
Standard asthma treatment
Anti- Inflamatory treatment:
Long term corticosteroids & Ibuoprofen –
Slow the progres of CF
30. Hemoptysis:
Minor hemoptysis- No specific treatment
Hemoptysis > 15-20ml
Admit in Ward
O2 Inhalation
Vit. K
Blood transfusion
Antibiotics
Stop chest physiotherapy
32. P E R T: (Lipase, Amylase, Proteases)
Fat absorption improves from 60% without
therapy to 85-90% with therapy
An upper limit of 10,000 units lipase/kg/day
PERT for infants 500-1000 U lipase/g of
dietary fat
PERT for children 500-4000 U lipase/g of
dietary fat
Fibrosing colonopathy
33. Nutritional Therapy:
*CF pts require 120% of normal energy
requirement
*Liberal diet containing
Fats, Sugar
Milk products, Protein foods
*Salt
< 6m
0.5g/d
6-12m
1.0g/d
1-5yrs
2.0g/d
>5yrs
3.0g/d
34. Treatment of other Complications:
1. Diabetes MellitusDKA rare
2. Musculoskeletal problemsPulmonary care & NSAID
3. Lung transplantationLife expectancy <2yrs
Quality of life impaired
Frequent life threatening
hemoptysis
35. Prognosis:
Median survival 40 yrs
> 90 % mortality is due to lung disease
Cirrhosis is second life threatening complication
Poor prognosis factors include
Female sex
Multiple organ involvement
Abnormal chest x- ray 12m after diagnosis
Poor growth
Recurrent hemoptysis
Pneumothorax
Corpulmonale
37. Pulmonary Treatment (Cont…)
Bronchodilator Treatment
Inhaled B2 agonists
Anticholinergics, Oral theophylline
Anti Inflammatory Treatment
Long term use of oral corticosteroids &
ibuprofen
Mucolytic Treatment:
Dornase Alpha (Recombinant Human
Deoxyribonuclease 1)
Cleaves extracellular DNA & decreases
viscoelasticity of purulent secretions
Used in chronic suppurative disease or
obstructive
disease
Dose: 2.5 mg OD
39. Pulmonary Treatment (Cont…)
Intravenous Antibiotic Therapy
Indicated in pts. Who have progressive
or unrelenting symptoms or signs
despite intensive home measures
Period of treatment is 14 days
Pseudomonas requires 2 drug therapy
40. The net ion flow across normal and cystic fibrosis
41. Other Diagnostic Tests
↑ potential difference across nasal epithelium
Loss of this difference with topical amiloride
application
Absence of voltage response to B–adrenergic
agonist
Failure to sweat when a combination of
isoproterenol and atropine is injected in
skin
42. Pathophysiology
CFTR mutation
In airways cells can’t excrete
Cl- from cells into the lumen
through cAMP mediated
CFTR channels
Cl- is trapped inside the cells
sodium follows chloride &
water follows sodium
secretions in the lumen
become viscous & elastic
& are harder to clear
Secretions are Retained
& obstruct airway
In sweat glands cAMP
mediated conductance of
Cl- in to the cells is defective
so Cl- can’ t be absorbed
from the sweat
sodium follows chloride
salt is lost in sweat
